Tre-P-23 - Papuloerythroderma of Ofuji successfully treated with oral retinoids

Abstract

Papuloerythroderma of Ofuji (PEO) is a rare skin disorder, which affects predominantly older males with male to female ratio 4:1. It is characterized by pruritic erythematous papules, which might progress to erythroderma. Exanthema typically spares the skin folds (deck-chair sign). Common laboratory findings include lymphopenia, peripheral eosinophilia and elevated serum IgE. Histological image generally shows non-specific inflammatory reaction and needs to be correlated with clinical and laboratory findings. PEO has been associated with cutaneous T-cell lymphomas (CTCL). However, this relationship remains poorly understood. Diagnosis of PEO should be established by exclusion and PEO individuals should be closely monitored for possible onset of CTCL. Case 1: A 85-year-old man with a 2-year history of pruritic non-confluent erythematous papules sparing the skin folds. Lymphopenia, eosinophilia and elevated serum IgE. Histology shows edematous changes of papillary dermis with mixed inflammatory infiltrate comprising multiple mast cells, eosinophils as well as isolated plasma cells. The diagnosis of PEO was made based on the typical clinical, laboratory and histopathological features. Case 2: A 95-year-old man with a 3-month history of pruritic maculopapular exanthema. Indolent multiple myeloma has been diagnosed 4 years prior to rash onset. Histology shows a non-specific spongiotic dermatitis with multiple eosinophils. In this context, eosinophilia found in the laboratory work-up allows to establish probable diagnosis of PEO. Pathogenesis of PEO is poorly elucidated and development of effective treatment guidelines thus remains challenging. As skin-homing Th2/Th22 cells seem to play a role in the pathogenesis of PEO, use of dupilumab, monoclonal antibody against IL-4Rα, has recently been investigated. We report a successful resolution in two patients with PEO using combination therapy of oral retinoids (acitretin, 10 mg/d) and local treatment (topical corticosteroids, tacrolimus, polidocanol). Our findings correlate well with recent reports suggesting higher success rates of oral retinoids compared to PUVA therapy or oral corticosteroids, yet with a more favourable safety profile and patient tolerance. Papuloerythroderma of Ofuji (PEO) is a rare skin disorder, which affects predominantly older males with male to female ratio 4:1. It is characterized by pruritic erythematous papules, which might progress to erythroderma. Exanthema typically spares the skin folds (deck-chair sign). Common laboratory findings include lymphopenia, peripheral eosinophilia and elevated serum IgE. Histological image generally shows non-specific inflammatory reaction and needs to be correlated with clinical and laboratory findings. PEO has been associated with cutaneous T-cell lymphomas (CTCL). However, this relationship remains poorly understood. Diagnosis of PEO should be established by exclusion and PEO individuals should be closely monitored for possible onset of CTCL. A 85-year-old man with a 2-year history of pruritic non-confluent erythematous papules sparing the skin folds. Lymphopenia, eosinophilia and elevated serum IgE. Histology shows edematous changes of papillary dermis with mixed inflammatory infiltrate comprising multiple mast cells, eosinophils as well as isolated plasma cells. The diagnosis of PEO was made based on the typical clinical, laboratory and histopathological features. A 95-year-old man with a 3-month history of pruritic maculopapular exanthema. Indolent multiple myeloma has been diagnosed 4 years prior to rash onset. Histology shows a non-specific spongiotic dermatitis with multiple eosinophils. In this context, eosinophilia found in the laboratory work-up allows to establish probable diagnosis of PEO. Pathogenesis of PEO is poorly elucidated and development of effective treatment guidelines thus remains challenging. As skin-homing Th2/Th22 cells seem to play a role in the pathogenesis of PEO, use of dupilumab, monoclonal antibody against IL-4Rα, has recently been investigated. We report a successful resolution in two patients with PEO using combination therapy of oral retinoids (acitretin, 10 mg/d) and local treatment (topical corticosteroids, tacrolimus, polidocanol). Our findings correlate well with recent reports suggesting higher success rates of oral retinoids compared to PUVA therapy or oral corticosteroids, yet with a more favourable safety profile and patient tolerance.